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Beckwith-Wiedeman syndrome with bilateral Wilms tumors

Case contributed by Ian Bickle
Diagnosis almost certain

Presentation

Polyhydramnios during pregnancy. Palpable left upper quadrant mass. Normal MRI within the last 8 months. Tumor?

Patient Data

Age: 18 months
Gender: Female

Performed 8 mth prior due...

mri

Performed 8 mth prior due to body asymmetry

Hemihypertrophy of the soft tissues of the thorax and abdomen.

The volume and size of the soft tissues are greater on the left than the right.

The kidneys are normal in appearance.

Ultrasound performed indicating a left sided renal mass.

Large solid enhancing mass occupying most of the left kidney.

Several lesions in the right kidney, the largest 1.7cm in the lower pole which is solid.

Hemihypertrophy of the torso.

Lungs clear.

Case Discussion

Beckwith-Wiedemann syndrome (BWS) is a congenital overgrowth disorder.  There is a significantly increased risk of malignancy, in particular, Wilms tumor.

The prior MRI during the first year of life showed normal kidneys.   Within less than a year bilateral Wilms tumors were evident.

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